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Prune Belly Syndrome (PBS)

PBS is a syndrome that occurs in about 1 in 29,000 to 40,000 live births and is much more common in boys.  It occurs during fetal development and is associated with urinary tract abnormalities, undescended testicles, and poorly developed abdominal muscles.  There may be other associated birth defects such as skeletal abnormalities, intestinal problems, and heart defects. Urinary tract abnormalities include a wide, elongated posterior urethra, dilated kidneys with varying degrees of kidney injury, large dilated tortuous ureters, and large bladders that may not function well.

Prenatal ultrasound findings include dilated ureters, a large bladder, and irregular abdominal wall.  Children may be born with very mild or very severe forms of prune belly.  Once a baby is born, various radiological and blood tests are done, and treatment is individualized to the baby’s needs.  Pediatric cardiologists, pulmonologists, and nephrologists are often involved in the management of these babies.  A procedure may be needed to ensure that the urine is drained and antibiotics are used to prevent infections.  Boys are often circumcised.  The testicles will eventually need to be brought down to the scrotum (orchidopexy), and the abdominal wall will need to be reconstructed in some boys with more severe abdominal muscle laxity. Bladder and kidney development is monitored lifelong.